Publications by authors named "Carol L Shields"

919 Publications

Secondary vasoproliferative tumor in adult-onset Coats disease.

Can J Ophthalmol 2021 Mar 31. Epub 2021 Mar 31.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa.

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http://dx.doi.org/10.1016/j.jcjo.2021.03.002DOI Listing
March 2021

Conjunctival melanoma: outcomes based on tumour origin in 629 patients at a single ocular oncology centre.

Eye (Lond) 2021 Mar 26. Epub 2021 Mar 26.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.

Purpose: To investigate clinical features and outcomes of conjunctival melanoma classified by tumour origin.

Methods: Retrospective review of conjunctival melanoma patients at a single ocular oncology centre between April 18, 1974 and September 9, 2019. Lesions were divided into three tumour origin groups (primary acquired melanosis [PAM], nevus, and de novo) and clinical features and outcomes were compared.

Results: There were 629 patients with conjunctival melanoma that arose from PAM (n = 476, 76%), nevus (n = 59, 9%), or de novo (n = 94, 15%). A comparison (PAM vs. nevus vs. de novo) revealed patients with tumours arising from PAM presented with older mean age (62 vs. 52 vs. 55 years, p < 0.001), worse initial logMAR visual acuity (Snellen equivalent 20/30 vs. 20/25 vs. 20/25, p = 0.03), and greater clock hour involvement (4.8 vs. 4.0 vs. 3.2, p < 0.001). Tumours arising from nevus had lower frequency of fornix (31% vs. 9% vs. 24%, p = 0.02) and tarsal involvement (29% vs. 9% vs. 26%, p = 0.046) and more frequent classification as AJCC category T1 (60% vs. 89% vs. 62%, p = 0.01). After follow-up of (57.2 vs. 68.2 vs. 51.7 months, p = 0.35), tumours arising from PAM had worse mean final visual acuity (20/50 vs. 20/40 vs. 20/40, p = 0.02) and greater frequency of visual acuity loss ≥3 lines (25% vs. 15% vs. 10%, p = 0.02). Kaplan-Meier estimates for 5-year risk showed no difference by tumour origin for visual acuity loss ≥3 lines, local tumour recurrence, exenteration, metastasis, or death.

Conclusions: Conjunctival melanoma most often arose from PAM, and tumour origin did not affect clinical outcomes.
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http://dx.doi.org/10.1038/s41433-021-01508-yDOI Listing
March 2021

An Echodense Choroidal Lesion in an Elderly Woman.

JAMA Ophthalmol 2021 Mar 25. Epub 2021 Mar 25.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1001/jamaophthalmol.2020.4642DOI Listing
March 2021

Multimodal imaging of a choroidal nevus with caverns in the setting of pachychoroid disease.

Retin Cases Brief Rep 2021 Feb 26. Epub 2021 Feb 26.

Doheny Eye Institute, Los Angeles, California, United States. Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles, California, United States. Eye Clinic, Department of Biomedical and Clinical Science "Luigi Sacco", Sacco Hospital, University of Milan, Milan, Italy. Department of Ophthalmology, Mayo Clinic, Rochester, MN, USA Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA Vitreous Retina Macula Consultants of New York, New York, New York. Department of Ophthalmology, NYU Grossman School of Medicine, New York, NY USA.

Purpose: To describe the multimodal imaging findings of extensive choroidal caverns within a choroidal nevus in an eye with pachychoroid spectrum disease.

Methods: A 69 year-old woman was referred with a known history of branch retinal vein occlusion (BRVO) in the right eye and choroidal nevus in the left eye. Fundus examination of both eyes (OU) revealed subretinal yellow deposits, suggestive of pachydrusen. Retinal venous collaterals were noted in the temporal macular of the right eye. A lightly pigmented choroidal lesion with nearly confluent overlying drusen and retinal pigment epithelial (RPE) alterations, consistent with chronic choroidal nevus, was noted in the macula of the left eye.

Results: Optical coherence tomography B-scans revealed thickened choroid (pachychoroid) with subfoveal choroidal thickness of 504 and 580 µm with large hyporeflective spaces suggestive of pachyvessels OU. In the region of the choroidal nevus, the choroidal vascular spaces appeared comparatively large and were classified as "caverns", measuring up to 480 µm in diameter. OCT angiography (OCTA) and indocyanine green angiography (ICGA) demonstrated absence of flow within the caverns. ICGA further illustrated choroidal vascular hyperpermeability with patchy hyperfluorescent areas OU. Widefield swept source OCT showed mild posterior scleral bowing, a feature occasionally documented with choroidal nevus, and highlighted greater hyporeflectivity and hypertransmission on OCT within the caverns compared to the non-cavernous choroidal vessels.

Conclusion: Choroidal caverns can occur within choroidal nevus in the setting of pachychoroid disease.
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http://dx.doi.org/10.1097/ICB.0000000000001138DOI Listing
February 2021

Aberrantly expressed GFRα-1/RET in patients with lacrimal adenoid cystic carcinoma is associated with high recurrence risk: a retrospective study of 51 LACC cases.

Cancer Biol Med 2021 Feb;18(1):199-205

Tianjin International Joint Research and Development Centre of Ophthalmology and Vision Science, Eye Institute and School of Optometry, Tianjin Medical University Eye Hospital, Tianjin 300384, China.

Objective: Because of the poor prognosis of lacrimal adenoid cystic carcinoma (LACC), we aimed to investigate the effects of perineural invasion (PNI) and consequent aberrations in GDNF/GFRα-1/RET protein expression on LACC recurrence.

Methods: Clinicopathological data for 51 histologically confirmed patients with LACC enrolled between 2001 and 2017 were retrospectively analyzed. Hematoxylin and eosin staining was applied to assess PNI. Tissue-based immunohistochemistry (IHC) detection of GDNF, GFRα-1, and RET proteins was performed on LACC formalin-fixed, paraffin-embedded specimens. We generated semi-quantitative data of the IHC results and compared them with the clinicopathological data for the 51 patients.

Results: Of the 51 patients, 19 (37.3%) were PNI positive. Recurrence was more common for LACC with than without PNI (73.7% 37.5%, = 0.01). GDNF, GFRα-1, and RET proteins were expressed in 62.7%, 62.7%, and 54.9% of the 51 patients with LACC, respectively. The expression of all 3 proteins was more common in patients with than without PNI. In agreement with previous findings, PNI-associated GFRα-1 and RET positivity, as detected by IHC, remained significantly associated with recurrence, whereas GDNF expression, as detected by IHC, was not correlated with LACC recurrence. Specifically, patients with concurrent GFRα-1 and RET expression may have a high risk of PNI (89.5% positivity rate) and recurrence (84.2% positivity rate).

Conclusions: PNI may contribute to LACC recurrence. The concurrent expression of GFRα-1 and RET proteins, as detected by IHC, may potentially be associated with LACC PNI and recurrence.
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http://dx.doi.org/10.20892/j.issn.2095-3941.2020.0271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7877180PMC
February 2021

Response criteria for intraocular retinoblastoma: RB-RECIST.

Pediatr Blood Cancer 2021 May 23;68(5):e28964. Epub 2021 Feb 23.

The Vision Center at Children's Hospital Los Angeles, Los Angeles, California, USA.

Standardized guidelines for assessing tumor response to therapy are essential for designing and conducting clinical trials. The Response Evaluation Criteria In Solid Tumors (RECIST) provide radiological standards for assessment of solid tumors. However, no such guidelines exist for the evaluation of intraocular cancer, and ocular oncology clinical trials have largely relied on indirect measures of therapeutic response-such as progression-free survival-to evaluate the efficacy of treatment agents. Herein, we propose specific criteria for evaluating treatment response of retinoblastoma, the most common pediatric intraocular cancer, and emphasize a multimodal imaging approach for comprehensive assessment of retinoblastoma tumors in clinical trials.
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http://dx.doi.org/10.1002/pbc.28964DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8049511PMC
May 2021

Primary treatment of ocular surface squamous neoplasia with topical interferon alpha-2b: Comparative analysis of outcomes based on original tumor configuration.

Indian J Ophthalmol 2021 Mar;69(3):563-567

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.

Purpose: The aim of this study was to evaluate tumor control of OSSN with topical IFNα2b alone based on tumor configuration (flat versus (vs.) dome-shaped).

Methods: Retrospective, nonrandomized, interventional cohort study on 64 consecutive tumors in 63 patients with OSSN treated with topical IFNα2b. Topical IFNα2b (1 million international units/cc) was compounded and provided by the Thomas Jefferson University Hospital Pharmacy to be refrigerated and applied 4 times daily until biomicroscopic evidence of tumor resolution was observed.

Results: The tumor configuration was flat (n = 15, 23%) or dome-shaped (n = 49, 77%). A comparison (flat vs. dome-shaped) revealed dome-shaped with older mean patient age at presentation (62 vs. 70 years, P = 0.04), greater patient history of smoking (13% vs. 42%, P = 0.04), greater corneal involvement (7% vs. 82%, P < 0.001), larger mean basal diameter (5.5 vs. 12.4 mm, P = 0.001) and mean thickness (1.9 vs. 4.3, P = 0.002), and longer mean duration IFNα2b therapy (3.7 vs. 6.3 months, P = 0.002). There was no difference in mean follow-up time (22.2 vs 23.1 months) or time to complete response (5.0 vs. 6.1 months). There was no difference in achievement of complete tumor control with IFNα2b alone (93% vs. 96%). There were no cases with metastasis or death.

Conclusion: Topical IFNα2b alone shows excellent overall tumor control of 95% with no difference in efficacy based on tumor configuration.
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http://dx.doi.org/10.4103/ijo.IJO_1665_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7942118PMC
March 2021

Overlapping Immunohistochemical Features of Adenocarcinoma of the Nonpigmented Ciliary Body Epithelium and Renal Cell Carcinoma.

Am J Ophthalmol 2021 Jan 30;226:191-200. Epub 2021 Jan 30.

From the David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.

Purpose: To find immunohistochemical markers that distinguish adenocarcinoma of the nonpigmented ciliary epithelium (NPCE) from metastatic carcinoma, especially metastatic renal cell carcinoma.

Design: Retrospective case series.

Methods: Three cases of adenocarcinoma of the NPCE were examined histologically with hematoxylin-eosin stain and immunohistochemical stains including vimentin, AE1/AE3, Cam 5.2, CK7, PAX2, PAX8, AMACR, and CAIX. We also reviewed previously reported cases of this tumor.

Results: We found that the immunohistochemical profile of adenocarcinoma of the NPCE can overlap with renal cell carcinoma. Both tumors can express vimentin, cytokeratin AE1/AE3, Cam 5.2, PAX2, PAX8, and AMACR. One of the adenocarcinomas of the NPCE in our series also expressed CD10 and the renal cell carcinoma marker (RCC Ma). Carbonic anhydrase IX (CAIX) was not detected in any of the 3 tumors.

Conclusions: Adenocarcinomas arising in phthisic eyes can be diagnostically challenging. We have found it particularly difficult to distinguish adenocarcinoma of the NPCE from metastatic carcinoma, especially metastatic clear cell renal cell carcinoma and papillary renal cell carcinoma. Because of the immunophenotypic overlap, most patients will require systemic workup including imaging of the kidneys to be certain of the diagnosis.
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http://dx.doi.org/10.1016/j.ajo.2021.01.020DOI Listing
January 2021

Presumed Natural History of Combined Hamartoma of the Retina and Retinal Pigment Epithelium.

Ophthalmol Retina 2021 Jan 28. Epub 2021 Jan 28.

UPMC Eye Center, University of Pittsburgh, Pittsburgh, Pennsylvania. Electronic address:

Purpose: To correlate structural changes of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with patient age.

Design: Retrospective study.

Participants: Fifty eyes of 49 patients (age range, 1-74 years) with CHRRPE studied at 9 tertiary vitreoretinal institutions.

Methods: We analyzed the clinical findings with respect to lesion topography and pigmentation as well as investigated the OCT findings regarding the thickness, vitreoretinal interface, outer plexiform layer distortion, ellipsoid zone disruption, and retinal pigment epithelium-Bruch's membrane complex involvement of CHRRPE.

Main Outcome Measures: Clinical and imaging findings of CHRRPE at different ages.

Results: Analysis of 50 CHRRPE patients revealed that younger patients were more likely to demonstrate partial thickness involvement of the retina (P = 0.009) with predominantly inner retinal layer involvement (P = 0.04). The inverse was true for older patients with CHRRPE. In addition, older patients more commonly showed pigmentary changes. Eyes with CHRRPE were more likely to show an increase in central macular thickness independently of tumor location.

Conclusions: Based on these findings, we believe that CHRRPE typically begins in the inner retina and continues toward the outer retina over time, with increase in central macular thickness, despite the location of the tumor.
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http://dx.doi.org/10.1016/j.oret.2021.01.011DOI Listing
January 2021

Outcomes of Intravenous Chemotherapy (Chemoreduction) for Retinoblastoma Based on Patient Age in 964 Eyes of 554 Patients.

Asia Pac J Ophthalmol (Phila) 2021 Jan 19. Epub 2021 Jan 19.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA Department of Ophthalmology, Mayo Clinic, Rochester, MN Department of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, PA.

Purpose: To evaluate retinoblastoma control after intravenous chemotherapy (chemoreduction) by patient age at presentation.

Design: Retrospective case series.

Methods: This study included 964 eyes of 554 patients treated with chemoreduction at Ocular Oncology Service at Wills Eye Hospital. Patients received 6 monthly cycles of standard chemoreduction. Additional therapies for tumor control were performed as needed.

Results: Of 964 eyes, a comparison by age group (<6 months vs. 6-12 months vs. 13-24 months vs. >24 months) revealed more advanced age group with higher frequency of group E tumor (15% vs. 25% vs. 32% vs. 39%, P < 0.001). By treatment outcomes, complete tumor control was achieved with chemoreduction alone more often in less advanced age group (46% vs. 30% vs. 17% vs. 8%, P < 0.001). Additional treatment after chemoreduction was needed more often in more advanced age group with external beam radiotherapy (EBRT; 9% vs. 16% vs. 20% vs. 15%, P = 0.006) or enucleation (12% vs. 18% vs. 26% vs. 37%, P < 0.001). Over time (1994-1998 vs. 1999-2003 vs. 2004-2008 vs. 2009-2013 vs. 2014-2019), the paradigm for additional required treatment after chemoreduction shifted toward less EBRT (27% vs. 24% vs. 14% vs. 7% vs. 2%, P < 0.001) and more intra-arterial (0% vs. 0% vs. 1% vs. 25% vs. 48%, P < 0.001) and intravitreal (0% vs. 0% vs. 3% vs. 10% vs. 20%, P < 0.001) chemotherapy.

Conclusions: Chemoreduction is a safe and effective treatment method for patients with retinoblastoma, demonstrating the best tumor control in the younger age groups.
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http://dx.doi.org/10.1097/APO.0000000000000360DOI Listing
January 2021

Pigmented Iris Lesion on Anterior-Segment Swept-Source Optical Coherence Tomography.

JAMA Ophthalmol 2021 01 20;139(1):e204079. Epub 2021 Jan 20.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1001/jamaophthalmol.2020.4079DOI Listing
January 2021

Reply.

Ophthalmology 2021 Apr 7;128(4):e23. Epub 2021 Jan 7.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1016/j.ophtha.2020.12.006DOI Listing
April 2021

Uveal Melanoma Metastasis to the Contralateral Eye Structures: A Retrospective Comparative Analysis of 13 Consecutive Patients.

Ophthalmol Retina 2021 Jan 7. Epub 2021 Jan 7.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address:

Purpose: To report uveal melanoma (UM) metastasis to the contralateral ocular and periocular structures.

Design: Retrospective study.

Participants: Thirteen patients with UM metastasis to the contralateral ocular and periocular structures were included.

Methods: Clinical records were reviewed retrospectively.

Main Outcome Measures: The development and time to onset of contralateral ocular and periocular metastasis, systemic metastasis, and death.

Results: Of the 13 000 treated UM patients, 13 patients were diagnosed with UM metastasis to the contralateral ocular and periocular structures. Mean patient age at primary UM diagnosis was 60 years (median, 60 years; range, 37-87 years). The primary uveal melanoma was in the choroid (n = 11) or ciliary body (n = 2) and was treated with brachytherapy (n = 11), proton beam radiotherapy (n = 1), or enucleation (n = 1). Systemic metastasis developed in 11 patients (85%) at a mean of 66 months (median, 34 months; range, 12-216 months) after diagnosis of the primary UM. All 11 patients (100%) showed liver metastasis and 8 patients (62%) also showed extrahepatic metastasis. The sites of metastasis to the contralateral ocular or periocular structures included the choroid in 4 patients (31%), the orbit in 7 patients (54%), and the eyelid in 2 patients (15%). One patient with eyelid metastasis demonstrated concurrent conjunctival nodule. Mean time to diagnosis of contralateral ocular or periocular metastasis was 94 months (median, 48 months; range, 9-375 months). Contralateral choroidal metastasis was multifocal in 3 of 4 patients (75%). Of 7 patients with orbital metastasis, 5 showed extraocular muscle involvement with restricted ocular motility. Treatment for contralateral choroidal metastasis included brachytherapy (n = 2), transpupillary thermotherapy (n = 1), and observation (n = 1). Treatment for contralateral periocular (orbit or eyelid) metastasis was excision (n = 5), external beam radiotherapy (n = 2), and observation (n = 2). Of 13 patients, death was documented in 11 patients at a mean of 17 months (median, 9 months; range, 3-54 months) as a result of systemic UM metastasis (n = 10) or unrelated cause (n = 1).

Conclusions: Metastasis resulting from UM to the contralateral ocular and periocular structures is rare and generally occurs in patients with disseminated metastasis. Orbital tissue is the most common site of involvement, and these patients have short life expectancy.
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http://dx.doi.org/10.1016/j.oret.2020.12.025DOI Listing
January 2021

A Hidden Pandemic.

AMA J Ethics 2020 12 1;22(12):E1067-1070. Epub 2020 Dec 1.

Director of the Ocular Oncology Service at Wills Eye Hospital in Philadelphia, Pennsylvania.

By March of 2020, the COVID-19 pandemic had changed our lives. Shadowing this pandemic is another one that adversely affects clinicians' educations and well-being. This article features a digital photo painting and commentary.
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http://dx.doi.org/10.1001/amajethics.2020.1067DOI Listing
December 2020

Boom-Boom Radiotherapy for Choroidal Lymphoma-Two Days and Done.

Ophthalmol Retina 2021 Jan;5(1):15

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1016/j.oret.2020.09.002DOI Listing
January 2021

Diffuse infiltrative retinoblastoma with retinal detachment in "cinnamon-roll" configuration: a case report.

J AAPOS 2021 Feb 15. Epub 2021 Feb 15.

Pathology Department, Mexican Social Security Institute Clinic No. 25, Monterrey, Mexico.

A 4-year-old girl presented with leukocoria, vision loss, and painless left eye redness. Posterior segment examination revealed a total retinal detachment and abnormal, tortuous retinal vessels in the left eye. No visible mass, vitreous seeding, or hemorrhage were observed. B-mode ultrasound showed a detached retina in a "cinnamon-roll" configuration. Due to poor visual prognosis and high clinical suspicion of malignancy, enucleation was performed. Further histopathological analysis confirmed the diagnosis of diffuse infiltrative retinoblastoma, with an atypical presentation.
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http://dx.doi.org/10.1016/j.jaapos.2020.10.004DOI Listing
February 2021

Conjunctival Melanoma: Outcomes based on the American Joint Committee on Cancer Clinical Classification (8th Edition) of 425 Patients at a Single Ocular Oncology Center.

Asia Pac J Ophthalmol (Phila) 2020 Dec 9;10(2):146-151. Epub 2020 Dec 9.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA.

Purpose: The aim of this study was to evaluate outcomes of conjunctiva melanoma based on the American Joint Committee on Cancer Classification (AJCC) 8th edition.

Design: Retrospective interventional case series.

Methods: Outcomes analysis of 425 patients.

Results: In this analysis of 425 patients with conjunctival melanoma, there were 266 (63%) patients classified as T1, 75 (18%) as T2, 84 (20%) as T3, and 0 (0%) as T4. A comparison (T1 vs T2 vs T3) revealed that history of primary acquired melanosis was more common in T2 (81% vs 96% vs 81%; P = 0.01) and conjunctival nevus more common in T1 (20% vs 9% vs 11%; P = 0.03). Of 381 patients with follow-up (mean of 57.6 months), comparison revealed higher T category with increasing local recurrence/new tumor (30% vs 43% vs 49%; P = 0.004), increasing exenteration (3% vs 9% vs 28%; P < 0.001), increasing melanoma-related locoregional lymph node metastasis (2% vs 7% vs 12%; P = 0.001), increasing melanoma-related systemic metastasis (9% vs 25% vs 23%; P < 0.001), and increasing melanoma-related death (4% vs 12% vs 18%; P < 0.001). A comparison at 10 years revealed visual acuity loss of >3 lines (32% vs 42% vs 63%; P < 0.001), melanoma recurrence/new tumor (47% vs 70% vs 74%; P < 0.001), exenteration (4% vs 24% vs 46%; P < 0.001), melanoma-related locoregional lymph node metastasis (3% vs 13% vs 25%; P < 0.001), melanoma-related systemic metastasis (13% vs 45% vs 40%; P < 0.001), and melanoma-related death (8% vs 22% vs 37%; P < 0.001).

Conclusions: Based on the AJCC 8th edition of conjunctival melanoma, the 10-year risk per T category significantly increased for visual acuity loss of >3 lines, recurrence/new tumor, exenteration, locoregional and systemic melanoma-related metastasis, and melanoma-related death.
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http://dx.doi.org/10.1097/APO.0000000000000343DOI Listing
December 2020

Ocular Surface Squamous Neoplasia Managed With Primary Interferon α2b: A Comparative Analysis of 212 Tumors in Smokers Versus Nonsmokers.

Cornea 2020 Dec 3. Epub 2020 Dec 3.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA.

Purpose: To explore clinical features and outcomes of ocular surface squamous neoplasia (OSSN) treated with primary interferon (IFN)-α2b, based on patient cigarette smoking status.

Methods: Retrospective nonrandomized, interventional cohort study on 212 consecutive tumors in 194 patients, all of whom were treated with topical and/or injection IFNα2b.

Results: There were 88 tumors in 76 patients with current or past smoking history (smokers) and 124 tumors in 118 nonsmoking patients (nonsmokers). A comparison (smokers vs. nonsmokers) revealed smokers with more frequent bilateral disease (16% vs. 3%, P = 0.003), more frequent involvement of inferior forniceal (34% vs. 21%, P = 0.03) and inferior tarsal conjunctiva (38% vs. 24%, P = 0.04), greater mean number of clock hour involvement (4.1 vs. 3.5 clock hours, P = 0.04), and greater dome growth pattern (30% vs. 15%, P = 0.01). There was no difference regarding method of IFNα2b administration as topical (61% vs. 71%, P = 0.14), injection (10% vs. 6%, P = 0.32), or combination topical/injection (28% vs. 23%, P = 0.33). A comparison revealed smokers with more frequent recurrence after initial response (23% vs. 13%, P = 0.04). There was no difference regarding initial tumor response or time to response, treatment side effects, or systemic outcomes.

Conclusions: Regarding ocular surface squamous neoplasia, smokers more often display bilateral, dome-shaped tumors with inferior forniceal or tarsal involvement, and greater extent than nonsmokers. After treatment with topical and/or injection IFNα2b, control is equivalent, but smokers show greater recurrence.
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http://dx.doi.org/10.1097/ICO.0000000000002615DOI Listing
December 2020

Painful Glaucoma and Pseudohypopyon in a Child with Retinoblastoma.

Ophthalmol Glaucoma 2020 Nov - Dec;3(6):480

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1016/j.ogla.2020.07.004DOI Listing
July 2020

Conjunctival melanoma: Risk factors for recurrent or new tumor in 540 patients at a single ocular oncology center.

Eur J Ophthalmol 2020 Nov 11:1120672120970393. Epub 2020 Nov 11.

Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.

Purpose: To investigate risk factors for recurrent or new tumor in patients with conjunctival melanoma.

Methods: Retrospective review of patients with conjunctival melanoma managed on the Ocular Oncology Service, Wills Eye Hospital from 1974 to 2019.

Results: There were 540 patients with mean follow-up of 57.6 months, of whom 176 (33%) had recurrent or new tumor formation. Risk factors for recurrent or new tumor on univariate analysis included presentation at older age (OR: 1.02 [1.01-1.03] per 1-year increase in age,  = 0.002), history of prior conjunctival surgery (OR: 1.62 [1.05-2.49],  = 0.03), worse visual acuity at presentation (OR: 1.76 [1.04-2.98] per 1 log-unit increase,  = 0.04), more advanced AJCC clinical T-subcategory (OR: 1.08 [1.02-1.14] per 1 subcategory increase,  = 0.01), tumor primary location in tarsal conjunctiva (OR: 1.80 [1.09-2.98],  = 0.02), and secondary tumor involvement of the fornix (OR: 1.68 [1.06-2.65],  = 0.03), and eyelid (OR: 1.92 [1.07-3.43],  = 0.03). Risk factors on multivariate analysis using all demographics, clinical features, and tumor location included presentation at older age (OR: 1.02 [1.00-1.03],  = 0.01), history of prior conjunctival surgery (OR: 1.84 [1.16-2.94],  = 0.01), and more advanced AJCC clinical T-subcategory (OR: 1.07 [1.01-1.13] per one subcategory increase,  = 0.03).

Conclusion: On multivariate analysis, the strongest predictors of recurrent or new tumor formation following treatment of conjunctival melanoma included older age, history of prior conjunctival surgery, and advanced AJCC T-subcategory. These results suggest that earlier detection and the first surgery in conjunctival melanoma management are critical for prevention of recurrent or new tumor, and we recommend prompt referral to an experienced surgeon.
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http://dx.doi.org/10.1177/1120672120970393DOI Listing
November 2020

Photodynamic Therapy in Ocular Oncology.

J Ophthalmic Vis Res 2020 Oct-Dec;15(4):547-558. Epub 2020 Oct 25.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, USA.

Over the past two decades, we have witnessed the increasing use of photodynamic therapy (PDT) in the field of ocular oncology. Based on a review of the literature and our own experience, we herein review the role of PDT for the management of intraocular tumors. The discussion includes two main topics. First, we discuss the application of PDT for benign tumors, including circumscribed choroidal hemangioma, choroidal osteoma, retinal astrocytoma, retinal capillary hemangioma (retinal hemangioblastoma), and retinal vasoproliferative tumor. Second, we assess the role of PDT for malignant tumors, including choroidal melanoma and choroidal metastasis.
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http://dx.doi.org/10.18502/jovr.v15i4.7793DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7591845PMC
October 2020

Retinoblastoma Vitreous Seeds Captured on OCT.

Ophthalmol Retina 2020 11;4(11):1117

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1016/j.oret.2020.05.004DOI Listing
November 2020

Validation of the Newly Proposed World Health Organization Classification System for Conjunctival Melanocytic Intraepithelial Lesions: A Comparison with the C-MIN and PAM Classification Schemes.

Am J Ophthalmol 2021 03 30;223:60-74. Epub 2020 Oct 30.

Department of Pathology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, USA; Department of Ophthalmology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

Purpose: We sought to compare the sensitivity, specificity, accuracy, and interobserver agreement of the two most commonly used classification systems for conjunctival melanocytic intraepithelial lesions with the new World Health Organization (WHO) classification.

Design: Retrospective case series and evaluation of classification systems.

Methods: We reviewed the pathology and medical records of all patients who underwent a primary biopsy procedure for conjunctival primary acquired melanosis (PAM) at Wills Eye Hospital between 1974 and 2002 who had ≥36 months of follow-up. Data collected included age, sex, clinical findings, recurrence, and progression to melanoma. Twelve ophthalmic pathologists analyzed scanned hematoxylin and eosin-stained virtual microscopic slides using 3 classification systems: PAM, conjunctival melanocytic intraepithelial neoplasia, and the WHO 4th edition classification of conjunctival melanocytic intraepithelial lesions. Observer agreement, sensitivity, specificity, and diagnostic accuracy of each classification system were assessed.

Results: There were 64 patients who underwent 83 primary excisions with cryotherapy for conjunctival PAM who had adequate tissue for histopathologic evaluation. The interobserver agreement in distinction between the low- and high-grade lesions was 76% for PAM, 67% for conjunctival melanocytic intraepithelial neoplasia, and 81% for WHO classification system. Low-grade lesions provided the greatest interpretative challenge with all 3 classification systems. The 3 classification systems had comparable accuracy of 81%-83% in their ability to identify lesions with potential for recurrence.

Conclusions: This study highlights the comparable strengths and limitations of the 3 classification systems for conjunctival melanocytic intraepithelial lesions and suggests that the simplified WHO classification scheme is appropriate for evaluation of these lesions.
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http://dx.doi.org/10.1016/j.ajo.2020.10.020DOI Listing
March 2021

Incidence and predictors of ophthalmic artery occlusion in intra-arterial chemotherapy for retinoblastoma.

J Neurointerv Surg 2020 Oct 29. Epub 2020 Oct 29.

Department of Neurological Surgery, Thomas Jefferson University - Center City Campus, Philadelphia, Pennsylvania, USA

Background: Retinoblastoma is the most common primary intraocular malignancy in children.

Objective: To determine the incidence of ophthalmic artery (OA) occlusion in patients treated with selective ophthalmic artery catheterization (OAC) for chemotherapy infusion for retinoblastoma. Also, to evaluate technical, anatomical, tumorous, and patient-related factors that are predictors of OA occlusion.

Methods: A retrospective chart review was performed for patients diagnosed with intraocular retinoblastoma and managed with intra-arterial chemotherapy (IAC).

Results: The total study cohort included 208 retinoblastoma tumors of 208 eyes in 197 consecutive patients who underwent 688 attempted IAC infusions overall with a total of 624 successful OAC infusions. The total incidence of ophthalmic artery thrombosis was 11.1% (23/208). The numbers of successful OAC procedures before diagnosing OA occlusion were one OAC in six cases (27.3%), two in seven cases (31.8%), three in four cases (18.2%), four in one case (4.5%), five in two cases (9.1%), and six in one case (4.5%).

Conclusions: Local factors relating to the chemotherapy and selective microcatheterization of the OA are essential factors in the development of OA thrombosis, as seen by the association of OA thrombosis with the frequency of IAC.
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http://dx.doi.org/10.1136/neurintsurg-2020-016759DOI Listing
October 2020

Modern treatment of retinoblastoma: A 2020 review.

Indian J Ophthalmol 2020 11;68(11):2356-2365

Ocular Oncology Service Wills Eye Hospital, Philadelphia, PA, USA.

Retinoblastoma management remains complex, requiring individualized treatment based on International Classification of Retinoblastoma (ICRB) staging, germline mutation status, family psychosocial factors and cultural beliefs, and available institutional resources. For this 2020 retinoblastoma review, PubMed was searched for articles dated as early as 1931, with an emphasis on articles from 1990 to the present day, using keywords of retinoblastoma, chemotherapy, intravenous chemotherapy, chemoreduction, intra-arterial chemotherapy, ophthalmic artery chemosurgery, intravitreal chemotherapy, intracameral chemotherapy, cryotherapy, transpupillary thermotherapy, laser, radiation, external beam radiotherapy, plaque radiotherapy, brachytherapy, and enucleation. We discuss current treatment modalities as used in the year 2020, including intravenous chemotherapy (IVC), intra-arterial chemotherapy (IAC), intravitreal chemotherapy (IvitC), intracameral chemotherapy (IcamC), consolidation therapies (cryotherapy and transpupillary thermotherapy [TTT]), radiation-based therapies (external beam radiotherapy [EBRT] and plaque radiotherapy), and enucleation. Additionally, we present a consensus treatment algorithm based on the agreement of three North American retinoblastoma treatment centers, and encourage further collaboration amongst the world's most expert retinoblastoma treatment centers in order to develop consensus management plans and continue advancement in the identification and treatment of this childhood cancer.
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http://dx.doi.org/10.4103/ijo.IJO_721_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7774148PMC
November 2020

Photopsia and visual loss in a patient with lung cancer.

Indian J Ophthalmol 2020 Nov;68(11):2348

Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, United States.

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http://dx.doi.org/10.4103/ijo.IJO_58_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7774178PMC
November 2020

Neglected Coats Disease.

J Pediatr Ophthalmol Strabismus 2020 Oct 20;57:e82-e85. Epub 2020 Oct 20.

Coats disease is a rare, non-hereditary retinal vascular abnormality that typically presents in the first two decades of life and is characterized by idiopathic retinal telangiectasia with progressive exudation. The authors describe a patient with Coats disease in which the family neglected treatment, demonstrating the natural course of this disease. [J Pediatr Ophthalmol Strabismus. 2020;57:e82-e85.].
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http://dx.doi.org/10.3928/01913913-20200818-01DOI Listing
October 2020

An Unusual Upper Eyelid Mass.

JAMA Ophthalmol 2020 12;138(12):1316-1317

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1001/jamaophthalmol.2020.2859DOI Listing
December 2020

Muir-Torre Syndrome Associated Periocular Sebaceous Neoplasms: Screening Patterns in the Literature and in Clinical Practice.

Ocul Oncol Pathol 2020 Aug 15;6(4):226-237. Epub 2020 Jan 15.

Department of Ophthalmic Pathology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

Background: Muir-Torre syndrome (MTS) is defined clinically as the association of cutaneous sebaceous neoplasm and visceral malignancy. Ancillary tests are considered crucial for diagnosis. Although screening guidelines for MTS, including the Mayo MTS scoring system, have been proposed, there are no ophthalmic site-specific guidelines.

Summary: A literature review conducted by PubMed search for articles describing patients with periocular sebaceous neoplasm and MTS disclosed 31 publications describing 60 patients, 36 (60%) of whom fulfilled clinical criteria for MTS, 6 (10%) whose diagnosis was based on screening ancillary studies, 14 (23%) who fulfilled clinical criteria and had supporting screening ancillary studies, and 4 (7%) who fulfilled clinical criteria and had supporting diagnostic genetic testing. Most patients were male (34 vs. 15 females), with a median age of 59 years (range 37-79 years). The most common diagnosis was sebaceous carcinoma (40/60, 67%), followed by sebaceous adenoma (16/60, 27%), followed by other tumors with sebaceous differentiation (4/60, 6%). The periocular lesions were identified prior to visceral malignancy in 10 out of 45 (22%) cases, after visceral malignancy in 34 out of 45 (76%) cases, and concurrently with visceral malignancy in 1 out of 45 (2%) cases. Immunohistochemistry for mismatch repair proteins was performed in 41 out of 60 (68%) and 14 out of 38 (37%) of the tumors had lost MSH2. Based on Mayo-MTS scores of 2 or greater, and after removing visceral malignancies not included in their scoring algorithm, 26 out of 30 of patients (87%) with complete data were considered to be appropriate candidates for further work-up. A survey of current practice was conducted by questionnaires, distributed to ophthalmic pathologists, ocular oncologists, and oculoplastic surgeons from national and international professional societies. Of the 103 physicians who participated in the survey, 91 (88%) felt that MTS evaluation guidelines were not sufficiently clear.

Key Messages: Our findings suggest that Mayo MTS screening guidelines may be applicable to periocular sebaceous neoplasms. The uncertainty of ophthalmic specialists about optimal screening guidelines for MTS reflects the heterogeneity of defining criteria for MTS and limited molecular genetic data. Larger studies with detailed clinical, histopathologic, and molecular genetic data are required to formally assess screening guidelines for MTS in patients with periocular sebaceous neoplasms.
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http://dx.doi.org/10.1159/000504984DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7506251PMC
August 2020

Usefulness of PAX8 Immunohistochemistry in Adult Intraocular Tumor Diagnosis.

Ophthalmology 2020 Sep 29. Epub 2020 Sep 29.

Ocular Oncology Service, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania.

Purpose: To evaluate the distribution of the PAX8 transcription factor protein in ocular tissues and to investigate if immunohistochemical stains for this biomarker are useful in the diagnosis of intraocular tumors.

Design: Observational case series.

Participants: Excision and cytologic analysis specimens of 6 ciliary body epithelial neoplasms, 2 iris epithelial neoplasms, 3 retinal pigment epithelial neoplasms, 3 intraocular medulloepitheliomas, 15 uveal melanomas, and 5 uveal melanocytomas.

Methods: Hematoxylin-eosin and PAX8 immunohistochemical stains were performed on all specimens. In appropriate cases, bleached preparations and other immunohistochemical stains, including AE1/AE3 cytokeratin, Lin28A, and CD45, were performed.

Main Outcome Measures: Distribution of PAX8 expression in normal and neoplastic tissue.

Results: Strong nuclear PAX8 expression was observed in the normal corneal epithelium, iris sphincter pupillae muscle, iris pigment epithelium and dilator muscle complex, nonpigmented and pigmented epithelia of the ciliary body, lens epithelium, and a subset of retinal neurons. The normal retinal pigment epithelium and uveal melanocytes did not stain for PAX8. The ciliary body epithelial and neuroepithelial tumors (adenoma, adenocarcinoma, and medulloepithelioma) showed uniform strong nuclear PAX8 immunoreactivity. All melanocytic tumors (iris melanoma, ciliary-choroidal melanoma, and melanocytoma) and retinal pigment epithelial neoplasms showed negative results for PAX8. A subset of tumor-associated lymphocytes, most prominent in uveal melanoma, showed positive results for PAX8. The uniformity of the PAX8 staining was superior to the variable cytokeratin staining in the ciliary epithelial neoplasms and the variable Lin28A staining in malignant medulloepithelioma. The veracity of PAX8 staining was equally as robust on cytologic analysis and open-flap biopsy specimens of ciliary epithelial and iris epithelial neoplasms, melanocytoma, and melanoma.

Conclusions: PAX8 has proven to be a very useful diagnostic marker in a select group of adult intraocular tumors, and we highly recommend its inclusion in diagnostic antibody panels of morphologically challenging intraocular neoplasms.
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http://dx.doi.org/10.1016/j.ophtha.2020.09.033DOI Listing
September 2020